Maintaining the thyroid gland in mutant thyroglobulin-induced hypothyroidism requires thyroid cell proliferation that must continue in adulthood.

ABSTRACT: Congenital hypothyroidism with biallelic thyroglobulin (Tg protein, encoded by the TG gene) mutation is an endoplasmic reticulum (ER) storage disease. Many patients (and animal models) grow an enlarged thyroid (goiter), yet some do not. In adulthood, hypothyroid TG^cog/cog mice (bearing a Tg-L2263P mutation) exhibit a large goiter, whereas adult WIC rats bearing the TG^rdw/rdw mutation (Tg-G2298R) exhibit a hypoplastic thyroid. Homozygous TG mutation has been linked to thyroid cell death, and cytotoxicity of the Tg-G2298R protein was previously thought to explain the lack of goiter in WIC-TG^rdw/rdw rats. However, recent studies revealed that TG^cog/cog mice also exhibit widespread ER stress-mediated thyrocyte death, yet under continuous feedback sti

SUBMITTER: Zhang X 

PROVIDER: S-EPMC9213252 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature